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Objective To assess the efficacy and safety of the IL-1b inhibitor canakinumab in all adults with refractory Still's disease identified from the National Organization For Medicines for off-label drug use. Methods : In a retrospective longitudinal multicenter cohort of 50 patients (median age 39 years) with active Still's disease despite treatment with corticosteroids (n = 11), conventional and synthetic (n = 34) and/or biologic disease modifying anti-rheumatic drugs (n = 30), we assessed the efficacy of canakinumab 150–300 mg administered every 4 (n = 47) or 8 weeks (n = 3) as combination therapy or monotherapy (n = 7) during a median follow-up of 27 (3–84) months. Results Α complete response was initially observed in 78% of patients within 3 months (median), irrespective of age at disease onset. A partial response was evident in 20%. One patient had resistant disease. Treatment de-escalation was attempted in 15 of 39 complete responders and a complete drug discontinuation in 21 patients for 8 months (median). Eleven patients (22%) relapsed during treatment, one during de-escalation process, and 11 after treatment discontinuation. Overall, 9 of 11 relapses were successfully treated with canakinumab treatment intensification or re-introduction. At last visit, 18% of patients were off treatment due to remission and 26% due to disease activity. Canakinumab had a significant corticosteroid sparing effect allowing weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient. Conclusion High rates of sustained remission were observed in this, largest so far, real-life cohort of adult patients with refractory Still's disease treated with canakinumab.

Background European data indicate that systemic sclerosis (SSc)-related death rates are increasing, thus raising concerns about SSc’s optimal management. Herein, we describe current treatment modalities and drug survival in a real-life SSc cohort. Methods Details on immunosuppressive/antiproliferative (methotrexate, mycophenolate, cyclophosphamide, azathioprine, rituximab, tocilizumab) and vasoactive agent [(endothelin receptor antagonists (ERAs), sildenafil, iloprost, and calcium channel blockers (CCB)] administration during the disease course (11.8 ± 8.4 years, mean + SD) of 497 consecutive patients examined between 2016 and 2018 were retrospectively recorded. Drug survival was assessed by Kaplan–Meier analysis. Results Methotrexate was the most frequently administered immunosuppressive/antiproliferative agent (53% of patients), followed by cyclophosphamide (26%), mycophenolate (12%), and azathioprine (11%). Regarding vasoactive agents, CCB had been ever administered in 68%, ERAs in 38%, iloprost in 7%, and sildenafil in 7% of patients; 23% of patients with pulmonary fibrosis had never received immunosuppressive/antiproliferative agents, 33% of those with digital ulcers had never received ERAs, iloprost, or sildenafil, whereas 19% of all patients had never received either immunosuppressive/antiproliferative or other than CCB vasoactive agents. Survival rates of methotrexate, cyclophosphamide, and mycophenolate differed significantly, being 84/75%, 59/43%, and 74/63% at 12/24 months, respectively, with inefficacy being the most frequent discontinuation cause. Conversely, CCB, ERAs, and sildenafil had high and comparable retention rates of 97/91%, 88/86%, and 80/80%, respectively. Conclusions Existing therapeutic limitations indicate that more evidence-based treatment is warranted for successful management of SSc. Vasculopathy seems to be managed more rigorously, but the low retention rates of immunosuppressive/antiproliferative drugs suggest that effective and targeted disease-modifying agents are warranted.

Objectives Evidence on comorbidity prevalence in antiphospholipid syndrome (APS) and its difference from high comorbidity burden rheumatic diseases is limited. Herein, we compare multiple comorbidities between APS and RA. Methods A total of 326 patients from the Greek APS registry [237 women, mean age 48.7 (13.4) years, 161 primary APS (PAPS), 165 SLE-APS] were age/sex matched (1:2 ratio) with 652 patients from a Greek multicentre RA cohort of 3115 patients. Prevalence of cardiovascular (CV) risk factors, stroke, coronary artery disease (CAD), osteoporosis, diabetes mellitus (DM), chronic obstructive pulmonary disease (COPD), depression and neoplasms were compared between APS and RA patients using multivariate regression analysis. Results Ηyperlipidemia and obesity (ΒΜΙ ≥ 30 kg/m2) were comparable while hypertension, smoking, stroke and CAD were more prevalent in APS compared with RA patients. Osteoporosis and depression were more frequent in APS, while DM, COPD and neoplasms did not differ between the two groups. Comparison of APS subgroups to 1:2 matched RA patients revealed that smoking and stroke were more prevalent in both PAPS and SLE-APS vs RA. Hypertension, CAD and osteoporosis were more frequent only in SLE-APS vs RA, whereas DM was less prevalent in PAPS vs RA. Hyperlipidaemia was independently associated with CV events (combined stroke and CAD) in PAPS and SLE-APS, while CS duration was associated with osteoporosis in SLE-APS. Conclusion Comorbidity burden in APS (PAPS and SLE-APS) is comparable or higher than that in RA, entailing a high level of diligence for CV risk prevention, awareness for depression and CS exposure minimization.

Background: European data indicate that systemic sclerosis (SSc)-related death rates are increasing, thus raising concerns about SSc's optimal management. Herein, we describe current treatment modalities and drug survival in a real-life SSc cohort. Methods: Details on immunosuppressive/antiproliferative (methotrexate, mycophenolate, cyclophosphamide, azathioprine, rituximab, tocilizumab) and vasoactive agent [(endothelin receptor antagonists (ERAs), sildenafil, iloprost, and calcium channel blockers (CCB)] administration during the disease course (11.8 ± 8.4 years, mean + SD) of 497 consecutive patients examined between 2016 and 2018 were retrospectively recorded. Drug survival was assessed by Kaplan-Meier analysis. Results: Methotrexate was the most frequently administered immunosuppressive/antiproliferative agent (53% of patients), followed by cyclophosphamide (26%), mycophenolate (12%), and azathioprine (11%). Regarding vasoactive agents, CCB had been ever administered in 68%, ERAs in 38%, iloprost in 7%, and sildenafil in 7% of patients; 23% of patients with pulmonary fibrosis had never received immunosuppressive/antiproliferative agents, 33% of those with digital ulcers had never received ERAs, iloprost, or sildenafil, whereas 19% of all patients had never received either immunosuppressive/antiproliferative or other than CCB vasoactive agents. Survival rates of methotrexate, cyclophosphamide, and mycophenolate differed significantly, being 84/75%, 59/43%, and 74/63% at 12/24 months, respectively, with inefficacy being the most frequent discontinuation cause. Conversely, CCB, ERAs, and sildenafil had high and comparable retention rates of 97/91%, 88/86%, and 80/80%, respectively. Conclusions: Existing therapeutic limitations indicate that more evidence-based treatment is warranted for successful management of SSc. Vasculopathy seems to be managed more rigorously, but the low retention rates of immunosuppressive/antiproliferative drugs suggest that effective and targeted disease-modifying agents are warranted. © 2020 The Author(s).

Giant cell arteritis (GCA) is the most common systemic vasculitis in the aged population associated with significant morbidity due to the long term administration of corticosteroids and the presence of various comorbidities. Data regarding its current treatment modalities, comorbidities, morbidity and mortality in Greece are limited. In this multi-center, prospective study that begun at the end of 2015 patients with newly diagnosed GCA according to the modified 1990 ACR criteria, as well as individuals with established or relapsing disease have been included. During the 1st phase of the study that is still ongoing, data are being collected concerning demographic and clinical characteristics of the patients, treatment at the onset of the disease and at relapses, relapses, adverse events of therapy, comorbidities, hospitalizations and deaths. During the 2nd and 3rd phase of the study patients will be reevaluated 2 and 5 years after their 1st evaluation. The study is expected to provide valuable data regarding the current clinical characteristics, co-morbidities, therapeutic regimens used, relapse rate, morbidity and mortality of patients with GCA.

Background: The pathophysiology of Systemic Sclerosis (SSc) encompasses autoimmunity, microvascular damage and fibrosis of the skin and internal organs. Accordingly, the two mainstays of treatment comprise antiproliferative and vasoactive regimens. So far there are limited data regarding the actual use of these agents in daily clinical practice. Objectives: To describe current treatment modalities based on standard practice decisions according to published recommendations and/or guidelines for SSc, as well as to determine to which extent different disease modifying agents, namely antiproliferative and vasoactive drugs, are administered to these patients. Methods: Consecutive patients from 7 academic rheumatology departments across Greece who fulfilled the 1980 american College of Rheumatology criteria for classification of SSc and were examined between January 2016 and December 2018 at least once, were included in the study. Patients’ medical records were analyzed and antiproliferative and vasoactive agents administered anytime during the disease course were recorded. Results: A total of 457 patients (87% women, 51% diffuse SSc, 62% with pulmonary fibrosis, 55% with digital ulcers, mean±SD age at diagnosis 49.2±13.1 years, disease duration 10.4±8 years) were studied. Methotrexate was the most frequent antiproliferative agent ever administered (53% of patients, 55% of them with diffuse SSc) followed by cyclophosphamide (26%, 78% of them diffuse), mycophenolate (11%, 63% of them diffuse), azathioprine (10%, 64% of them diffuse), rituximab (9%, 84% of them diffuse) and tocilizumab (3%, 68% of them diffuse). Among vasoactive agents, endothelin receptor antagonists (ERA) were most frequently ever administered in 39% of patients (64% with diffuse SSc) followed by iloprost in 7% (55% diffuse) and 5 phosphodiesterase (5PDE) inhibitors in 5.5% of patients (62% diffuse). Among all antiproliferative/vasoactive agents used, the greatest retention rate at last follow-up visit since initiation had ERA (82%) and 5PDE inhibitors (86%). Of note, 20% of SSc patients (90% women, 70% limited SSc, age at diagnosis 50.4±13.2 years, disease duration 8.4±6.4 years) had never been treated with either antiproliferative or vasoactive agents, whereas 41% had ever received only antiproliferative and 8% only vasoactive therapy. Moreover, 1/5 of patients with pulmonary fibrosis had never received antiproliferative treatment, whereas 1/3 of patients with digital ulcers had never received vasoactive drugs (Table). Conclusion: About 20% of contemporary SSc patients, including patients with pulmonary fibrosis and digital ulcers, have never received antiproliferative or vasoactive therapy, possibly reflecting the spectrum of benign disease and/or a subgroup of under-treated patients. References: Disclosure of interests: None declared

Background: Giant cell arteritis (GCA) usually requires long-term therapy with corticosteroids and/or Disease Modifying Anti-rheumatic Drugs (DMARDs) but the exact factors that are associated with drug discontinuation in these patients have not been well defined. Objectives: To evaluate the baseline or on-treatment factors that influence drug survival in GCA. Methods: Data were derived from an ongoing, multicenter, prospective cohort study of patients with GCA. During the 1st phase of the study, data regarding demographic and clinical characteristics at baseline, type of treatment, adverse events of therapy and co-morbidities were retrospectively collected and analyzed. Predictors of drug discontinuation were examined by univariate and multivariate logistic regression analyses. Results: One hundred and seventy seven patients were included in the study; 70% (n=120) were women with a mean age at diagnosis of 74 ± 8.4 years. Diagnosis was established by temporal artery biopsy in 127 patients (72%), ultrasound of temporal arteries in 37 patients (21%) and large vessel imaging in 10 patients (6%). At diagnosis, the median ESR and CPR were 103 mm/h and 63 mg/L, respectively. All patients were treated initially with pos corticosteroids (median daily starting dose: 41 mg), while 12 (7%) of patients received pulse steroids. At the 1st patient evaluation (median follow-up from diagnosis: 3 years), the median daily steroid dose was 5 mg, while in 34% (n= 62) and 8% (n= 14) of patients a synthetic or biologic DMARD had been added, respectively. During that period, 24% (n=43) of patients had discontinued corticosteroids and 18% (n=32) all treatments. By univariate analysis, DMARD use was associated with a higher possibility for corticosteroid discontinuation (OR=2.3, p=0.017) but by multivariate logistic regression analysis only female sex (OR=0.49, p=0.07) and age at diagnosis (OR=0.94, p=0.01) were associated with a decreased risk for corticosteroid discontinuation. Similar results were found for discontinuation of all drugs [female sex (OR=0.4, p=0.05) and age at diagnosis (OR=0.9, p=0.046)]. Conclusion: In this large GCA cohort, only one out of four patients managed to discontinue corticosteroids and 20% all treatments ∼3 years after diagnosis. Multivariate analysis revealed only female sex and age at diagnosis as independent factors for treatment discontinuation. Acknowledgement: Supported by grants from the Greek Rheumatology Society and Professional Association of Rheumatologists. Disclosure of Interests: None declared

Background Comorbidities are common in chronic systemic connective tissue diseases and are associated with adverse outcomes, increased morbidity and mortality. Although the prevalence of comorbidities has been well-studied in isolated diseases, comparative studies between different autoimmune diseases are limited. In this study, we compared the prevalence of common comorbidities between patients with systemic sclerosis (SSc) and patients with rheumatoid arthritis (RA). Methods Between 2016 and 2017, 408 consecutive patients with SSc, aged 59 ± 13 years (87% women), were matched 1:1 for age and gender with 408 patients with RA; mean disease duration was 10 ± 8 and 9 ± 8 years, respectively. Rates of cardiovascular risk factors, coronary artery disease, stroke, chronic obstructive pulmonary disease (COPD), osteoporosis, neoplasms and depression were compared between the two cohorts. Results The prevalence of dyslipidemia (18.4% vs 30.1%, p = 0.001) and diabetes mellitus (5.6% vs 11.8%, p = 0.007) and body mass index (p = 0.001) were lower in SSc compared to RA, while there was no difference in arterial hypertension or smoking. While there was a trend for lower prevalence of ischemic stroke in SSc than in RA (1.1% vs 3.2%, p = 0.085), coronary artery disease was comparable (2.7% vs 3.7%). No differences were found between patients with SSc and patients with RA in the prevalence of COPD (5.2% vs 3.7%), osteoporosis (24% vs 22%) or neoplasms overall (1.1% vs 1.7%); however lung cancer was the most prevalent cancer in SSc (7/17, 41%), whereas hematologic malignancies (7/19, 36%) and breast cancer (7/19, 36%) predominated in RA. Depression was more prevalent in SSc (22% vs 12%, p = 0.001), especially in diffuse SSc. Conclusions Despite the prevalence of dyslipidemia and diabetes mellitus in SSc being almost half that in RA, the cardiovascular comorbidity burden appears to be similar in both. The overall prevalence of neoplasms is no higher in SSc than in RA, but SSc has a more negative impact on quality of life, as clearly, more SSc patients develop depression compared to patients with RA.

Background:Golimumab is a tumor necrosis inhibitor (TNFi) approved for the treatment of axial SpA (axSpA) and psoriatic arthritis (PsA), both falling under the Spondyloarthritis (SpA) domain. Real-world data regarding its effect on work productivity (WP) and activity impairment (AI) are limitedObjectives:To assess the impact of golimumab on WP and AI over 12 months of treatment in patients with SpA, overall, and in the axSpA and PsA subpopulationsMethods:A 12-month non-interventional, multicenter, prospective study performed in the routine clinical care. Data were collected at baseline (BL: prior to treatment onset), 3, 6 and 12 months. Adult work-active consented patients with axSpA [ankylosing spondylitis (AS) or non-radiographic axSpA (nr-axSpA)] or PsA, newly initiated on golimumab as per approved label, were concequetively enrolled by 20 sites. Patients prior in >1 biologic agent, or switched from another TNFi due to primary non-response or safety were excluded. WP and AI was assessed with the Work Productivity and Activity Impairment: Specific Health Problem (WPAI:SHP) instrumentResults:Between Apr-2017 and May-2018, 121 (51: PsA, 70: axSpA) eligible patients (mean age: 45.4 years; 49.6% males; 69.0% overweight/obese; median disease duration: 11.3 months), (Figure 1), were enrolled. Median study duration participation: 11.9 months. Overall, 60.3% of the patients had previously received disease-modifying antirheumatic drugs and 16.5% biologics. At BL, the mean (standard deviation: SD) DAS28-ESR of the SpA population and PsA and axSpA subpopulations was 4.0 (1.3), 4.5 (1.2), and 3.6 (1.2), while the mean (SD) BASDAI score of patients with axSpA was 5.6 (1.9). At BL 94.1 and 96.7% of the SpA population reported WP loss and AI due to their SpA respectively, and at 3 months 87.3, and 88.0% respectively. In SpA population, the median BL WP loss and AI were 70.0% and 65.0% and decreased by a median of 31.4% and 40.0% at 3 months, by 44.2% and 40.0% at 6 months and by 50.0% and 50.0% at 12 months, respectively (Table 1). Improvements in WP loss and AI were noted in patients with PsA, axSpA, AS and nr-axSpA (Table 1). 12-month golimumab retention rate: 91.7%. No new safety signals emergedTable 1.Decreases from BL at 3, 6 and at 12 months in WP loss and overall AI with the WPAI:SHP instrumentWP loss (%)AI (%)Decrease from BL,median (n)Decrease from BL,median (n)3 months6 months12 months3 months6 months12 monthsOverall SpA populationa31.4a(n=102)44.2a(n=94)50.0a(n=87)40.0a(n=107)40.0a(n=101)50.0a(n=92)PsA31.4b(n=46)51.4a(n=42)53.6a(n=40)40.0a(n=47)50.0a(n=44)60.0a(n=40)axSpA33.0b(n=56)30.4b(n=52)45.5b(n=47)40.0a(n=60)40.0b(n=57)40.0b(n=52)ASc25.1 (n=35)29.9 (n=32)39.8 (n=29)20.0 (n=39)30.0 (n=37)30.0 (n=34)nr-axSpAc47.4 (n=21)55.4 (n=20)53.2 (n=18)50.0 (n=21)55.0 (n=20)50.0 (n=18)aSignificant decreases (pbSignificant decreases (pcStatistical significance of the change from baseline was not examined due to the small observations’ numberConclusion:Patients in the SpA population and axSpA and PsA subpopulations treated with golimumab in a routine care setting experienced significant improvements in work productivity and daily activities at 3, 6 and 12 months after treatment initiationAcknowledgments:The authors thank the following investigators: Ampatziadis E., Voulgari P., Gazi S., Georgiou P., Georgountzos A., Karokis D., Mpotzoris V., Mpournazos E., Sakkas L., Sidiropoulos P., and Vassilopoulos D. The study was Sponsored by MSD, Greece.Disclosure of Interests:Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Anastassios Kotrotsios Grant/research support from: MSD, Novartis, Roche, Consultant of: Bristol Myers Squibb, UCB pharma, Speakers bureau: Genesis pharma, UCB pharma, MSD, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Andreas Bounas Grant/research support from: MSD, AbbVie, Novartis, Genesis pharma, Consultant of: MSD, Bristol-Myers Squibb, UCB pharma, AbbVie, Speakers bureau: MSD, Bristol-Myers Squibb, Pfizer, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, GEORGIOS VOSVOTEKAS Grant/research support from: MSD, Janssen, Consultant of: MSD, Novartis, Roche, UCB pharma, Bristol-Myers Squibb, AbbVie, Speakers bureau: UCB pharma, Menarini, Bristol-Myers Squibb, MSD, Evangelia Petrikkou Employee of: MSD, Bristol Myers Squibb, Vianex SA, Gkikas Katsifis Grant/research support from: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Consultant of: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche, Speakers bureau: UCB Pharma, Janssen, Abbvie, Novartis, MSD, Aenorasis, Genesis Pharma, Pfizer, Roche

Background:Comorbidities in rheumatic diseases (RDs) have been associated with increased morbidity and mortality. Evidence on prevalence of comorbidities in antiphospholipid syndrome (APS) and its difference from high comorbidity burden RDs is limited.Objectives:To compare the prevalence of common comorbidities between APS [primary (PAPS) and Systemic lupus erythematosus (SLE)-APS] and Rheumatoid arthritis (RA) patients.Methods:326 APS patients from the Greek registry (237 women, mean age 48.7±13.4 years, 161 PAPS) were matched 1:2 for age and sex with 652 RA patients from Greek RA Registry. Prevalence of cardiovascular (CV) risk factors, stroke, coronary artery disease (CAD), osteoporosis, diabetes mellitus (DM), Chronic obstructive pulmonary disease (COPD), depression and neoplasms were compared between APS and RA using logistic regression analysis.Results:Regarding CV burden, hyperlipidemia and obesity (ΒMI≥30) were comparable while hypertension, smoking, CAD and stroke were more prevalent in APS compared to RA patients (Table 1). Osteoporosis and depression were more frequent in APS while DM, COPD and neoplasms were comparable between two groups. Comparison of APS subgroups to 1:2 matched RA patients revealed that smoking and stroke were more prevalent in PAPS and SLE-APS vs RA. Hypertension, CAD and osteoporosis were more prevalent only in SLE-APS vs. RA while DM was less prevalent in PAPS vs. RA patients.Table 1.Comparison of comorbidities between Antiphospholipid syndrome (APS) vs. matched Rheumatoid Arthritis (RA) patients and between primary APS (PAPS) or Systemic Lupus Erythematosus-APS (SLE-APS) vs matched RA patientsAPSRAOR*PAPSRAORSLE-APSRAORn (%)326652161322165330Hypertension97 (29.8)136 (21)1.61 (1.19-2.18)40 (25)75 (23.3)1.09 (0.70-1.69)57 (34.6)61 (18.5)2.33 (1.52-3.56)Smoking175 (53.7)264 (40.5)1.70 (1.30-2.22)87 (54)142 (44)1.49 (1.02-2.18)88 (53.3)122 (37)1.95 (1.33-2.85)Hyperlipidemia79 (24.2)135 (20.7)1.23 (0.89-1.68)40 (24.8)62 (19.3)1.39 (0.88-2.18)39 (23.6)73 (22)1.09 (0.70-1.70)Obesity48 (20.5)105 (19.5)1.06 (0.73-1.56)20 (17)51 (19)0.86 (0.49-1.52)28 (24)54 (19.7)1.28 (0.76-2.15)Stroke±66 (20.3)9 (1.4)13.8 (6.5-29.1)36 (22.4)4 (1.2)19.9 (6.6-59.9)30 (18.2)5 (1.5)7.8 (2.7-22.6)Coronary disease±16 (4.9)13 (2)3.14 (1.17-8.45)2 (1.2)7 (2.2)0.46 (0.04-4.77)14 (8.5)6 (1.8)10.9 (2.7-44.3)Osteoporosis×66 (20.3)92 (14)1.45 (1.01-2.06)19 (11.8)42 (13)0.96 (0.54-1.73)47 (28.5)50 (15)1.91 (1.20-3.05)Diabetes×18 (5.5)58 (9)0.58 (0.33-1.01)5 (3)29 (9)0.34 (0.13-0.89)13 (8)29 (9)0.88 (0.44-1.79)COPD≠11 (3.4)14 (2.2)1.26 (0.56-2.84)3 (1.9)6 (2)0.96 (0.23-4.0)8 (5)8 (2.4)1.28 (0.44-3.72)Depression#53 (16.3)66 (10)1.70 (1.15-2.53)23 (14)30 (9.3)1.69 (0.93-3.05)30 (18.2)36 (10.9)1.65 (0.96-2.84)Neoplasms˅14 (4.3)27 (4.1)1.05 (0.54-2.06)5 (3)12 (3.7)0.84 (0.28-2.52)9 (5.5)15 (4.6)1.31 (0.55-3.1)*OR: Odds ratio, crude or adjusted for: ± age, sex, smoking, hypertension, hyperlipidemia, BMI, corticosteroid (Cs) duration × Cs duration ≠ smoking, Cs duration #sex, disease duration, Cs duration ˅ age, disease durationConclusion:Comorbidity burden in APS (PAPS and SLE-APS) is comparable or even higher to that in RA, entailing a high level of diligence for CV risk prevention, awareness for depression and corticosteroid exposure minimization.Disclosure of Interests:Stylianos Panopoulos: None declared, Konstantinos Thomas: None declared, Georgios Georgiopoulos: None declared, Dimitrios Boumpas Grant/research support from: Unrestricted grant support from various pharmaceutical companies, Christina Katsiari: None declared, George Bertsias Grant/research support from: GSK, Consultant of: Novartis, Alexandros Drosos: None declared, Kyriaki Boki: None declared, Theodoros Dimitroulas: None declared, Alexandros Garyfallos Grant/research support from: MSD, Aenorasis SA, Speakers bureau: MSD, Novartis, gsk, Charalambos Papagoras: None declared, PELAGIA KATSIMPRI: None declared, Apostolos Tziortziotis: None declared, Christina Adamichou: None declared, Evripidis Kaltsonoudis: None declared, Evangelia Argyriou: None declared, GEORGIOS VOSVOTEKAS Grant/research support from: MSD, Janssen, Consultant of: MSD, Novartis, Roche, UCB pharma, Bristol-Myers Squibb, AbbVie, Speakers bureau: UCB pharma, Menarini, Bristol-Myers Squibb, MSD, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB, Dimitrios Vassilopoulos: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer

Background:Interleukin-1 (IL-1) is a major mediator of the inflammatory cascade in Still’s disease and an established therapeutic target.Objectives:To assess the efficacy and safety of the IL-1b inhibitor canakinumab in adolescent and adult patients with refractory Still’s disease.Methods:We conducted a retrospective longitudinal outcome study of 50 consecutive patients aged 39 years (median, range 14-72), fulfilling the Yamaguchi disease classification criteria, with active disease despite treatment with corticosteroids (CS) (n=11) and/or methotrexate (n=9) and/or biologics (n=30) [tumor necrosis factor inhibitors (n=13), IL-6 blockade (n=7), abatacept (n=2), anakinra (n=24); ≥1 biologics (n=13)]. Canakinumab 150-300 mg was administered sc, starting every 4 (n=48) or 8 weeks (n=2), for a median of 24 months (range 3-84). Concomitant treatment included CS (n=41), methotrexate (n=12) and leflunomide (n=3).Results:Complete remission was initially achieved in 78% of patients within a median time of 3 months, irrespective of age at disease onset. Partial clinical and laboratory response was evident in 20%. Canakinumab was discontinued in one patient with resistant disease (primary failure) and in 6 out of 10 initial responders, who relapsed during treatment (secondary failure). Of 39 patients in complete remission, increase in drug administration interval and/or drug dose reduction was attempted in 7, of which only 1 relapsed, whereas drug discontinuation was attempted in 19 patients for a median time of 8 months (range 3-68), of which 8 relapsed. Overall, in half of all disease flares, canakinumab re-introduction or intensification was successful. Canakinumab had a significant CS sparing effect permitting weaning in 21 of 41 cases. Infections (20%, severe 4%) and leucopenia (6%) led to treatment cessation in one patient.Conclusion:In this largest so far real-life patient cohort with refractory Still’s disease, high rates of sustained remission were induced by canakinumab both in adolescent and adult patients.Disclosure of Interests:Katerina Laskari: None declared, Panagiotis Athanassiou Grant/research support from: MSD, Genesis pharma, Janssen, Consultant of: Roche, Genesis pharma, Janssen, Speakers bureau: MSD, Janssen, Roche, Genesis pharma, Athanasios Georgiadis: None declared, Charalampos Gerodimos: None declared, Georgia Gkoni: None declared, Dimitrios Daoussis: None declared, Theodoros Dimitroulas: None declared, Despoina Dimopoulou: None declared, Chrysoula Iliou: None declared, Ioannis Kallitsakis Grant/research support from: MSD, Speakers bureau: Genesis pharma, Bristol-Myers Squibb, Dimitrios Karamitsos: None declared, Christina Katsiari: None declared, Stamatis-Nick Liossis: None declared, Clio Mavragani: None declared, CHARALAMPOS PAPAGORAS: None declared, Dimitrios Pikazis: None declared, Ioannis Raftakis: None declared, Theodosios Sarikoudis: None declared, Loukas Settas: None declared, Prodromos Sidiropoulos: None declared, Despoina Soukera: None declared, Evangelos Theodorou: None declared, Panagiota Tsatsani: None declared, Eleni Tsiakou: None declared, Dimitrios Vassilopoulos: None declared, PANAYIOTIS VLACHOYIANNOPOULOS: None declared, Georgios Vosvotekas: None declared, Paraskevi V. Voulgari: None declared, Marina Zakalka: None declared, Maria Tektonidou Grant/research support from: AbbVie, MSD, Novartis and Pfizer, Consultant of: AbbVie, MSD, Novartis and Pfizer, Petros Sfikakis Grant/research support from: Grant/research support from Abvie, Novartis, MSD, Actelion, Amgen, Pfizer, Janssen Pharmaceutical, UCB

Background An increased burden of comorbid conditions negatively impacts patients’ outcomes, leads to increased mortality and seems to characterise all chronic systemic connective tissue diseases. Systemic Sclerosis (SSc) is associated with the highest mortality rate comparing to other diseases, whereas data regarding epidemiology and clinical expression of SSc comorbidities is limited. In contrast, comorbidities of rheumatoid arthritis (RA), and especially the increased rate of cardiovascular disease, are better established. Objectives To compare the prevalence of common comorbidities in SSc versus RA in a large multicentre case-control study from 5 academic centres in Greece. Methods Between 2016 and 2017 consecutive SSc patients (n=408, mean age: 58.6 years, 88% women) were matched 1:1 for age and gender with 408 RA patients. Evaluated comorbidities were dyslipidemia, diabetes mellitus, arterial hypertension, coronary artery disease, stroke, chronic obstructive pulmonary disease, osteoporosis, neoplasms and depression. Differences were examined by x2 test. Results The prevalence of dyslipidemia (18.4% vs 30.1%, p=0.001) and diabetes mellitus (5.6% vs 11.8%, p=0.007) was lower in SSc than RA patients and there was no difference regarding arterial hypertension (31.8% vs 30.6%, respectively, p=0.742) between the two groups. Disease duration, smoking and alcohol consumption were comparable between SSc and RA groups. While there was a trend for lower prevalence of ischaemic strokes in SSc than RA (0.4% vs 2.2%, p=0.085), comparable rates of coronary artery disease were noted (2.7% v.s 3.7%, p=0.445). No differences were found between SSc and RA patients regarding chronic obstructive pulmonary disease (5.2% vs 3.7%, respectively. p=0.326), osteoporosis (24% vs 22%, p=0.668) and neoplasms (1.1% vs 1.7%, p=0.534). Depression requiring treatment was more prevalent in SSc compared to RA patients (22% vs 12%, p=0.001). Conclusions Despite almost half prevalence of dyslipidemia and diabetes mellitus in SSc versus RA patients, the cardiovascular comorbidity burden appears to be similar between the two diseases. SSc has no higher prevalence of neoplasms than RA but a greater negative impact on quality of life, as clearly more SSc patients develop depression compared to RA patients. Acquisition of prospective data is currently underway. Disclosure of Interest None declared

BACKGROUND: Comorbidities are common in chronic systemic connective tissue diseases and are associated with adverse outcomes, increased morbidity and mortality. Although the prevalence of comorbidities has been well-studied in isolated diseases, comparative studies between different autoimmune diseases are limited. In this study, we compared the prevalence of common comorbidities between patients with systemic sclerosis (SSc) and patients with rheumatoid arthritis (RA). METHODS: Between 2016 and 2017, 408 consecutive patients with SSc, aged 59 ± 13 years (87% women), were matched 1:1 for age and gender with 408 patients with RA; mean disease duration was 10 ± 8 and 9 ± 8 years, respectively. Rates of cardiovascular risk factors, coronary artery disease, stroke, chronic obstructive pulmonary disease (COPD), osteoporosis, neoplasms and depression were compared between the two cohorts. RESULTS: The prevalence of dyslipidemia (18.4% vs 30.1%, p = 0.001) and diabetes mellitus (5.6% vs 11.8%, p = 0.007) and body mass index (p = 0.001) were lower in SSc compared to RA, while there was no difference in arterial hypertension or smoking. While there was a trend for lower prevalence of ischemic stroke in SSc than in RA (1.1% vs 3.2%, p = 0.085), coronary artery disease was comparable (2.7% vs 3.7%). No differences were found between patients with SSc and patients with RA in the prevalence of COPD (5.2% vs 3.7%), osteoporosis (24% vs 22%) or neoplasms overall (1.1% vs 1.7%); however lung cancer was the most prevalent cancer in SSc (7/17, 41%), whereas hematologic malignancies (7/19, 36%) and breast cancer (7/19, 36%) predominated in RA. Depression was more prevalent in SSc (22% vs 12%, p = 0.001), especially in diffuse SSc. CONCLUSIONS: Despite the prevalence of dyslipidemia and diabetes mellitus in SSc being almost half that in RA, the cardiovascular comorbidity burden appears to be similar in both. The overall prevalence of neoplasms is no higher in SSc than in RA, but SSc has a more negative impact on quality of life, as clearly, more SSc patients develop depression compared to patients with RA.